Fatal systemic air embolism in a neonate with absent aortic valve.

A 32-year-old pregnant woman was referred at 33 weeks' gestation for prenatal ultrasound demonstrating fetal hydrops due to absent aortic valve with free aortic valve insufficiency. Elective caesarian section at 34 week's gestation was performed. Surgical intervention was planned immediately after labor at which time mitral valve closure and atrial septostomy using cardiopulmonary bypass would be performed. However, before insertion of the cannula for cardiopulmonary bypass, a gush of air from the right atrium was noted. The surgical procedure was abandoned because systemic air embolism was suspected. The child died 2 h after birth. Autopsy showed absent aortic valve with closed foramen ovale and left-ventricular hypertrophy. Microscopic findings showed pulmonary and systemic lymphangiectasis, which caused the introduction of air into systemic venous system by way of lymphatic duct just after birth.

Outcomes of cardiac surgery in trisomy 18 patients.

OBJECTIVE: The objective was to clarify the outcomes of cardiac surgery in trisomy 18 patients. PATIENTS AND METHODS: We analysed 34 consecutive trisomy 18 patients, of whom 21 were males, with cardiac complications. They were divided into patients who underwent cardiac surgery and those who were conservatively treated. We compared rates of survival and discharge alive between two groups. RESULTS: The surgery group included nine patients, with six males, who underwent cardiac surgery - intracardiac repair in three patients, pulmonary arterial banding in five patients, and ligation of the ductus in one patient - at median age of 2.2 months, ranging from 0.5 to 9.8, and with median weight of 2.6 kilograms, ranging from 1.5 to 3.2. Cardiac surgery and pre-operative assisted ventilation were hazardous factors leading to death. In the surgery group, cumulative survival rates at 1 month, 6 months, 12 months, and 24 months were 63%, 38%, 25%, and 22%, respectively, compared with 51%, 26%, 9%, and 9% in the conservative group. There was a significant difference (p = 0.002). The cumulative rates of discharge alive at 1 month, 3 months, and 6 months were 0%, 12%, and 65% in the surgery group, which did not differ from the conservative group (p = 0.80). CONCLUSIONS: Cardiac surgery contributed to increased survival rate but not the rate of discharge alive in trisomy 18 patients. Cardiac surgery could not prevent all the trisomy 18 patients from death. The indication of cardiac surgery should be carefully individualised to improve the quality of life in trisomy 18 patients and concerned surrounding people.

Exertional oscillatory ventilation during cardiopulmonary exercise test in Fontan patients with total cavopulmonary connection.

Exertional oscillatory ventilation (EOV) has been noted during cardiopulmonary exercise testing (CPX) in patients with heart failure. EOV is a predictor of poor prognosis in adult patients with heart failure. The objective of this study was to clarify the incidence and influence of EOV in Fontan patients. Symptom-limited CPX was performed in 36 Fontan patients at 12.3 +/- 4.3 (6.5-24.4) years of age or 5.9 +/- 2.0 (3.0-11.2) years after total cavopulmonary connection (TCPC). Mean age at the time of TCPC was 6.3 +/- 3.3. All 36 TCPC patients were classified as New York Heart Association classification I or II. They also underwent cardiac catheterization subsequently. EOV was defined as cyclic fluctuations in minute ventilation at rest that persist during effort lasting >or=60% of the exercise duration, with an amplitude >or=15% of the average resting value. EOV was noted in 21 of 36 Fontan patients (58%) with good clinical status. Univariable analysis between Fontan patients with and those without EOV showed significant differences in age at TCPC (p < 0.05), age at CPX (p < 0.02), weight at CPX (p < 0.02), follow-up duration between TCPC and CPX (p < 0.04), ventricular morphology (p < 0.05), and metabolic equivalents (p < 0.05) and peak minute oxygen uptake (VO(2)) per body weight (p < 0.05). Multivariable analysis showed that EOV was significantly related to peak VO(2) per kilogram. In conclusion, EOV was frequently noted during exercise in Fontan patients with good clinical status. EOV during exercise seems to be related to higher peak VO(2) per kilogram and younger age at TCPC, which is a contrary result to those for adult patients with chronic heart failure. EOV is a remarkable phenomenon during exercise to compensate for impaired cardiopulmonary function in Fontan patients.

Ventricular mechanics in the bidirectional glenn procedure and total cavopulmonary connection.

BACKGROUND: The time course of ventricular efficiency in Fontan candidates who underwent both the bidirectional Glenn procedure (BDG) and total cavopulmonary connection (TCPC) were analyzed in this study. We previously reported that volume-load reduction of BDG preceding TCPC allowed for any afterload mismatch to be corrected, thereby improving ventricular efficiency after staged TCPC. METHODS: We measured percent normal systemic ventricular end-diastolic volume (%N-EDV), contractility (end-systolic elastance [Ees]), afterload (effective arterial elastance [Ea]), and ventricular efficiency (ventriculoarterial coupling [Ea/Ees]) based on cardiac catheterization data before and after both BDG and staged TCPC in 30 patients. Ees and Ea were approximated as follows: Ees = mean arterial pressure/minimal ventricular volume, and Ea = maximal ventricular pressure/(maximal ventricular volume - minimal ventricular volume), and Ea/Ees was then calculated. Ventricular volume was divided by body surface area. RESULTS: The %N-EDV decreased both after BDG and after staged TCPC, thus resulting in an improvement of Ees. Although Ea increased both after BDG and after staged TCPC, Ea decreased during the interval between BDG and staged TCPC. These changes resulted in an improvement in Ea/Ees during the interval period and after staged TCPC, although Ea/Ees worsened after BDG. CONCLUSIONS: Correction of afterload mismatch during the interval period between BDG and staged TCPC is considered to be one of the most important factors for obtaining excellent clinical results when selecting a staged strategy to treat high-risk Fontan candidates.

Primary tissue failure of the Freestyle stentless aortic root bioprosthesis.

We present a case of early tissue failure of the Medtronic Freestyle stentless bioprosthesis used in the aortic position which led to reoperation 27 months after implantation. Almost one half of the left coronary cusp of the prosthetic valve had torn away along the annulus. The tear started from the left-non coronary commissure and reached the midpoint of the base of the left coronary cusp. The prosthetic valve was easily excised and replaced with a new mechanical valve. There was no sign of calcification or vegetation in the excised valve. The cause of the valve dysfunction was thought to be primary tissue failure. Long-term function of the Freestyle stentless valve should be carefully followed up.

Haemostatic profile of small children during and following cardiopulmonary bypass.

OBJECTIVE: We evaluated changes of the haemostatic system during pediatric cardiac surgery during and after cardiopulmonary bypass (CPB). METHOD: Twenty-five children under 15 kg of body weight undergoing open-heart surgery were divided into three groups; 9 patients (Group A), no bank blood was used throughout the surgery; 8 patients (Group B), packed red cells were used in the priming of CPB circuit; 8 patients (Group C) in cyanotic condition, for whom surgery was performed without bank blood. CPB caused a significant decrease of platelet counts in all three groups, the levels of which remained similar next morning. RESULTS: Platelet counts decreased more significantly in Group C (59+/-27 k/mm3) than in Group A (119+/-42 k/mm3) and B (104+/-27 k/mm3). Platelet function-platelet activating factor test (HemoSTATUS) did not significantly decrease throughout the perioperative period in Group A. HemoSTATUS value decreased during CPB and recovered after CPB in Group B and C. Prothrombin time international ratio (PT-INR) and activated partial thromboplastin time were significantly prolonged just after CPB and recovered until next morning in all three groups. PT-INR was more prolonged in Group C (2.92+/-0.62) than in Group A (2.08+/-0.27) and B (2.42+/-0.42). There was no significant difference in postoperative bleeding for the first 12 hours among the three groups. CONCLUSION: Although extreme hemodilution during CPB significantly impairs the coagulation and platelet system, these changes are usually transient and tolerable with minimal postoperative hemorrhage. However, a prolonged CPB and preoperative cyanotic condition may induce a critical decrease of platelet counts and increase postoperative bleeding.

Effect of biventricular pacing therapy in patients with dilated cardiomyopathy with severe congestive heart failure.

OBJECTIVES: Biventricular pacing (BVP) therapy has recently emerged as an effective treatment for patients with moderate to severe congestive heart failure (CHF) and ventricular asynchrony all over the world. However, this therapy is not yet available in Japan. We evaluated the effects of BVP in patients with severe CHF due to dilated cardiomyopathy (DCM). SUBJECTS: Four patients with medically refractory severe CHF due to DCM in New York Heart Association functional class III or IV heart failure underwent BVP therapy. We combined the implantation of the left ventricular (LV) epicardial lead via small thoracotomy following right atrial and ventricular intravenous leads under general anesthesia. We evaluated to determine whether improvements of ventricular function, ventricular size, mitral regurgitation, functional status, frequency of hospitalization, and quality of life were associated with BVP therapy. RESULTS: BVP improved LV systolic function, decreased LV size and mitral regurgitation, and shortened prolonged QRS interval. The patients' symptoms, exercise tolerance, frequency of hospitalization, and quality of life were also dramatically improved by BVP. Furthermore, combination of BVP and oral administration of amiodarone significantly prevented recurrence of ventricular tachycardia and paroxysmal atrial fibrillation, and maintained sinus rhythm for a long period. CONCLUSIONS: In view of these findings, BVP therapy may contribute to the development of new therapeutic method for patients with severe CHF due to DCM.

One-stage operation for esophageal perforation of a thoracic aortic aneurysm.

The patient was a 65-year-old man. Preoperative computed tomography showed a ruptured thoracic aortic aneurysm that formed a submucosal hematoma in the thoracic esophagus with perforation near the esophageal-cardiac junction. A one-stage operation was performed. The aortic arch and proximal descending aorta were replaced with rifampicin-soaked synthetic grafts, followed by subtotal esophagectomy with primary reconstruction using a gastric tube. His early postoperative course was uneventful, and he started oral intake on postoperative day (POD) 15; however, dysphagia occurred on POD 20, and an esophageal fistula and mediastinitis developed more than 1 month after the operation. The patient recovered from mediastinitis after 4 months of mediastinal drainage and administration of antibiotics. Thus, a one-stage operation for esophageal perforation of a ruptured thoracic aortic aneurysm with primary esophageal reconstruction is possible in selected patients. Care must be taken to avoid postoperative compression of the reconstructed esophagus by a mediastinal hematoma.

Longitudinal growth of the autologous vessels above and below the Gore-Tex graft after the extracardiac conduit Fontan procedure.

OBJECTIVE: Reflecting excellent mid-term outcomes, extracardiac conduit Fontan procedure (ECFP) using Gore-Tex tube graft has been performed with increasing frequency in patients with functional single ventricle. Nevertheless, due to the lack of growth potential of the artificial conduit, the status of the venous pathway along with somatic growth is a continuing concern. In this study, we evaluated the longitudinal growth of the autologous vessels above and below the Gore-Tex graft used in the ECFP. METHODS: This study included 34 patients who had completed cardiac catheterisations at 1 month and 5.1 years after the ECFP. The average age, weight and height at the ECFP were 3.8+/-2.5 years (1.8-12.7 years), 12.7+/-4.6 kg (7.4-33.0 kg) and 92.9+/-16.1cm (72.5-153.5 cm), respectively. We measured the vertical lengths of three different parts angiographically: the length between the confluence point of the innominate vein and the anastomotic site of the conduit to the pulmonary artery (SVC-C), the conduit vertical length (C) and the length between the confluence point of the hepatic vein and the conduit's anastomotic site to the inferior vena cava (IVC-C). RESULTS: We have not observed stenosis or thrombus formation in the conduit or distortion of the conduit or pulmonary artery in any of the cases. No intervention or re-operation related to the extracardiac conduit was required, and laminar flow through the conduit was maintained with efficient Fontan haemodynamics. At 5.1 years after the ECFP, the average weight and height gain were 10.3+/-4.4 kg and 28.5+/-1.9 cm, respectively. The length of SVC-C, C and IVC-C were significantly increased as 124+/-15%, 106+/-7% and 132+/-24%, respectively, compared to the lengths at 1 month after the ECFP. The degree of increase in SVC-C and IVC-C was significantly larger than that in C. CONCLUSIONS: Along with the patient's somatic growth, longitudinal growth of the autologous vessels above and below the Gore-Tex graft was demonstrated to compensate for the lack of growth potential of the artificial graft.

Catastrophic hemorrhage due to aortic injury during resternotomy: what to do?

Aortic injury occurred during resternotomy in a 22-year-old woman who had undergone a Rastelli-type operation. Although extracorporeal circulation using the right femoral artery and vein was commenced immediately, hemostasis through the midline incision was impossible, and her circulation was hardly maintained. Thus, the parasternal approach was attempted, dividing the right first through fourth ribs and the head of the right clavicle. This approach enabled the assistant to give effective compression to the aortic injury site. Dissection of the adhesions around the ascending aorta was safely carried out, and the aortic laceration was closed without the help of circulatory arrest.

Clinical results of commissure plication annuloplasty for mitral regurgitation in children.

PURPOSE: We evaluated the clinical results of commissure plication annuloplasty for mitral regurgitation (MR) in children. METHODS: Twenty-eight patients underwent a valve repair with commissure plication annuloplasty for MR from 1988 to 2005. The mean age was 2.7 +/- 3.3 years. Several appropriate techniques were combined (cleft closure in 5 patients, chordal shortening in 2 patients, artificial chordal replacement in 4 patients, leaflet fixation in 2 patients, and so on). The mean follow-up period was 6.2 years. RESULTS: There was one operative death (3.6%) and no late deaths. Two patients underwent a second repair 19 and 23 months after their initial repairs. The actuarial freedom from the reoperation rate was 90.4% +/- 0.6% at 10 years. The freedom from moderate MR or more was shown to decrease over time, 87.8% +/- 0.7% at 5 years and 78.0% +/- 11.0% at 10 years. Furthermore, the 10-year freedom from mild MR or more was 56.5% +/- 11.9%. A progression of MR was seen. Most of the residual or recurrent MR cases weighed less than 10 kg at operation. CONCLUSIONS: The combination of commissure plication annuloplasty and several appropriate techniques provided adequate results for MR in children. Since a progression of MR was observed, a careful follow-up is therefore needed in such cases.

Mid-term follow-up of the status of Gore-Tex graft after extracardiac conduit Fontan procedure.

OBJECTIVE: Extracardiac conduit Fontan procedure (ECFP) using Gore-Tex graft has been performed with increasing frequency for the patients with functional single ventricle. However, lack of growth potential and longevity of the conduit are consistent concerns and main points of criticism of the ECFP. In this study, we investigated the mid-term status of the Gore-Tex graft used in the ECFP by comparing the internal diameter of the graft with the inferior vena cava (IVC) diameter at 1 month and 5.2 years after the ECFP. METHODS: Of 79 patients who underwent ECFP using Gore-Tex graft between November 1997 and December 2007, 33 patients who had completed cardiac catheterization at 1 month (21-73 days) and 5.2 years (3.3-9.6 years) after the ECFP were included in this study. We measured the internal diameter of the Gore-Tex graft and IVC at both catheterizations retrospectively. RESULTS: The size of the Gore-Tex graft used in the ECFP was 16 mm in 17 patients, 18 mm in 9 patients, and 20mm in 7 patients. Laminar flow through the conduits was maintained without any stenosis or kinking of the graft in these 33 patients. No intervention or reoperation related to the extracardiac conduit has been required. There were no significant differences in mean cross-sectional area (CSA) of the conduits at 1 month versus 5.2 years after the ECFP for each conduit size, and no significant changes in the conduit-to-IVC CSA ratio (0.98+/-0.40 vs 0.82+/-0.21 for 16 mm, 1.09+/-0.30 vs 0.92+/-0.33 for 18 mm, and 1.16+/-0.55 vs 0.94+/-0.44 for 20mm conduit). CONCLUSIONS: The conduit CSA and conduit-to-IVC CSA ratio remained unchanged in small caliber grafts down to 16 mm at 5.2 years after the ECFP. However, further investigation is necessary to evaluate the fate of the Gore-Tex graft and late hemodynamics in the patients with small conduits after they achieve full somatic growth.

Surgical results of anomalous origin of the right pulmonary artery from the ascending aorta including reoperation for infrequent complications.

BACKGROUND: We evaluated the results of surgery for an anomalous origin of the right pulmonary artery from the ascending aorta. METHODS: From August 1986 to December 2005, 8 children (6 neonates) aged 7 to 180 days (mean, 35 +/- 59 days) with anomalous origin of the right pulmonary artery from the ascending aorta underwent surgical repair at our institute. All except one child, who had the distal form, had the proximal form. Cardiac catheterization showed that the left pulmonary artery to systemic pressure ratio was 1.0 or more. Surgery was performed by direct anastomosis in 7 patients and by graft interposition in 1. RESULTS: There were no operative or late deaths. All patients postoperatively underwent cardiac catheterization that showed decreased left pulmonary artery to systemic pressure ratio ranging from 0.2 to 0.6. Follow-up periods ranged from 2 months to 13 years. We undertook reoperations for two infrequent postoperative causes. One patient exhibited significant supravalvar aortic stenosis and required patch enlargement of the ascending aorta 3 years after operation. The other patient (with the distal form) needed a reoperation after 1 month because of progressive stenosis at the anatomic site. Graft interposition was performed, and histopathologic examination showed that the tissue from the stenotic region looked like that of a ductus. CONCLUSIONS: We undertook surgical repair for anomalous origin of the right pulmonary artery from the ascending aorta. Pulmonary hypertension was improved in all patients. Careful follow-up was necessary to detect supravalvar aortic and anastomotic stenosis early and late after operation.

Redirection of the hepatic venous flow for the treatment of pulmonary arteriovenous malformations after Fontan operation.

A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.

Long-term effectiveness of total arch replacement for type A aortic dissection.

BACKGROUND: With recent improvements in cerebral protection during aortic arch repair, total aortic arch replacement has become an accepted surgical method for acute type A aortic dissection involving the aortic arch. Our surgical strategy is to perform total arch replacement with a branched graft using antegrade selective cerebral perfusion for the patients with type A aortic dissection involving the aortic arch. The objective of this study is to evaluate the effectiveness of this strategy on late outcome. METHODS: From October 1988 to April 2003, 46 patients underwent total arch replacement for acute type A dissection involving the aortic arch. Operations were performed with hypothermic cardiopulmonary bypass, antegrade selective cerebral perfusion during the arch repair, and open distal anastomosis. RESULTS: Hospital mortality was 6.5% (3 patients), and permanent neurologic dysfunction was observed in 1 patient. During the follow-up period (mean, 5.4 years; range, 13 months to 15.6 years), 2 patients died, but the causes were not related to the aorta or aortic valve. Survival rates at 5 and 10 years postoperatively were 89.6% +/- 5.2% and 82.7% +/- 8.2%, respectively. Of the 41 survivors, 3 patients underwent successful reoperation for the distal thoracic aorta. Freedom from reoperation was 93.6% +/- 4.6% and 88.7% +/- 6.5% at 5 and 10 years, respectively. The residual false lumen in the thoracic aorta was frequently thrombosed (76.2%). CONCLUSIONS: Total arch replacement for acute type A dissection may decrease the risk of late complications related to the false lumen and lead to excellent long-term survival.